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Osteosarcoma
Andrew T. Trout, MD
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Pathology

          • Clinical Issues

            TERMINOLOGY

            • Abbreviations

              • Osteosarcoma (OS)
            • Synonyms

              • Osteogenic sarcoma
              • Telangiectatic OS: Hemorrhagic OS, malignant bone aneurysm, aneurysmal bone cyst-like OS
              • Low-grade central OS: Low-grade intramedullary OS, well-differentiated intramedullary OS, low-grade intraosseous-type OS
              • Parosteal OS: Juxtacortical OS, juxtacortical low-grade OS, low-grade surface OS
            • Definitions

              • Primary malignant tumor of bone (mesenchymal origin) with cells producing osteoid
              • WHO subtypes
                • Conventional OS (~ 75%)
                • Telangiectatic OS (5-7%)
                • Small cell OS (1-2%)
                • Low-grade central OS (1-2%)
                • Secondary OS
                • Surface OS
                  • Parosteal OS (5%)
                  • Periosteal OS (1-2%)
                  • High-grade surface type (1%)
              • Location
                • Appendicular > > > axial skeleton (~ 10%)
                • ~ 90% of conventional OS is metaphyseal
                • ~ 75% of periosteal OS is diaphyseal
                • Majority of OS occurs in femur (40-70%)
                  • Proximal tibia (16-25%) and proximal humerus (5-15%) are next most common for conventional OS
                • Rarely soft tissue primary
              • Morphology
                • Aggressive osseous tumor with variable lytic and osteoblastic components and variable associated soft tissue mass
                  • Depending on tumor subtype and histologic variation
            • Associated Syndromes

              • Predisposition with
                • Retinoblastoma gene mutation
                • Li-Fraumeni syndrome
                • Rothmund-Thompson syndrome
                • Mutations of TP53 and MDM2 (murine double minute oncogene)
                • Chromosomal heterozygosity at 3q, 13q, and 18q

            IMAGING

            • F-18 FDG PET/CT

              • PET/MR

                • F-18 NaF PET Bone Scan

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          Selected References

                                          1. Hurley C et al: Comparison of (18) F-FDG-PET-CT and bone scintigraphy for evaluation of osseous metastases in newly diagnosed and recurrent osteosarcoma. Pediatr Blood Cancer. 63(8):1381-6, 2016
                                          2. Kubo T et al: Prognostic significance of (18)F-FDG PET at diagnosis in patients with soft tissue sarcoma and bone sarcoma; systematic review and meta-analysis. Eur J Cancer. 58:104-11, 2016
                                          3. Liu F et al: Performance of positron emission tomography and positron emission tomography/computed tomography using fluorine-18-fluorodeoxyglucose for the diagnosis, staging, and recurrence assessment of bone sarcoma: a systematic review and meta-analysis. Medicine (Baltimore). 94(36):e1462, 2015
                                          4. Quartuccio N et al: Pediatric bone sarcoma: diagnostic performance of ¹⁸F-FDG PET/CT versus conventional imaging for initial staging and follow-up. AJR Am J Roentgenol. 204(1):153-60, 2015
                                          5. Green JT et al: Osteogenic tumors of bone. Semin Diagn Pathol. 31(1):21-9, 2014
                                          6. Luetke A et al: Osteosarcoma treatment - where do we stand? A state of the art review. Cancer Treat Rev. 40(4):523-32, 2014
                                          7. Byun BH et al: Combination of 18F-FDG PET/CT and diffusion-weighted MR imaging as a predictor of histologic response to neoadjuvant chemotherapy: preliminary results in osteosarcoma. J Nucl Med. 54(7):1053-9, 2013
                                          8. Fox MG et al: Osteosarcoma: review of the various types with emphasis on recent advancements in imaging. Semin Musculoskelet Radiol. 17(2):123-36, 2013
                                          9. Hongtao L et al: 18F-FDG positron emission tomography for the assessment of histological response to neoadjuvant chemotherapy in osteosarcomas: a meta-analysis. Surg Oncol. 21(4):e165-70, 2012
                                          10. Inaki A et al: Thallium-201 scintigraphy for the assessment of long-term prognosis in patients with osteosarcoma. Ann Nucl Med. 26(7):545-50, 2012
                                          11. Cui J et al: Epigenetic changes in osteosarcoma. Bull Cancer. 98(7):E62-8, 2011
                                          12. Dai X et al: Review of therapeutic strategies for osteosarcoma, chondrosarcoma, and Ewing's sarcoma. Med Sci Monit. 17(8):RA177-190, 2011
                                          13. Kaste SC: Imaging pediatric bone sarcomas. Radiol Clin North Am. 49(4):749-65, vi-vii, 2011
                                          14. Yarber JL et al: Targeted therapies in bone sarcomas: current approach and future directions. Expert Opin Investig Drugs. 20(7):973-9, 2011
                                          15. Gorlick R et al: Osteosarcoma. J Bone Miner Res. 25(4):683-91, 2010
                                          16. Ritter J et al: Osteosarcoma. Ann Oncol. 21 Suppl 7:vii320-5, 2010
                                          17. Cheon GJ et al: Prediction model of chemotherapy response in osteosarcoma by 18F-FDG PET and MRI. J Nucl Med. 50(9):1435-40, 2009
                                          18. Costelloe CM et al: 18F-FDG PET/CT as an indicator of progression-free and overall survival in osteosarcoma. J Nucl Med. 50(3):340-7, 2009
                                          19. Gorlick R: Current concepts on the molecular biology of osteosarcoma. Cancer Treat Res. 152:467-78, 2009
                                          20. Ottaviani G et al: The etiology of osteosarcoma. Cancer Treat Res. 152:15-32, 2009
                                          21. Damron TA et al: Osteosarcoma, chondrosarcoma, and Ewing's sarcoma: National Cancer Data Base Report. Clin Orthop Relat Res. 459:40-7, 2007
                                          22. McCarville MB et al: The cause and clinical significance of central tumor photopenia on thallium scintigraphy of pediatric osteosarcoma of the extremity. AJR Am J Roentgenol. 188(2):572-8, 2007
                                          23. Nadel HR: Bone scan update. Semin Nucl Med. 37(5):332-9, 2007
                                          24. Hayden JB et al: Osteosarcoma: basic science and clinical implications. Orthop Clin North Am. 37(1):1-7, 2006
                                          25. Bacci G et al: Neoadjuvant chemotherapy for osteosarcoma of the extremities with metastases at presentation: recent experience at the Rizzoli Institute in 57 patients treated with cisplatin, doxorubicin, and a high dose of methotrexate and ifosfamide. Ann Oncol. 14(7):1126-34, 2003
                                          26. Brenner W et al: PET imaging of osteosarcoma. J Nucl Med. 44(6):930-42, 2003
                                          27. Ozcan Z et al: Correlation of 99mTc-sestamibi uptake with blood-pool and osseous phase 99mTc-MDP uptake in malignant bone and soft-tissue tumours. Nucl Med Commun. 22(6):679-83, 2001
                                          28. Garcia R et al: Comparison of fluorine-18-FDG PET and technetium-99m-MIBI SPECT in evaluation of musculoskeletal sarcomas. J Nucl Med. 37(9):1476-9, 1996
                                          Related Anatomy
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                                          Related Differential Diagnoses
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                                          References
                                          Tables

                                          Tables

                                          KEY FACTS

                                          • Terminology

                                            • Imaging

                                              • Top Differential Diagnoses

                                                • Pathology

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Osteosarcoma (OS)
                                                    • Synonyms

                                                      • Osteogenic sarcoma
                                                      • Telangiectatic OS: Hemorrhagic OS, malignant bone aneurysm, aneurysmal bone cyst-like OS
                                                      • Low-grade central OS: Low-grade intramedullary OS, well-differentiated intramedullary OS, low-grade intraosseous-type OS
                                                      • Parosteal OS: Juxtacortical OS, juxtacortical low-grade OS, low-grade surface OS
                                                    • Definitions

                                                      • Primary malignant tumor of bone (mesenchymal origin) with cells producing osteoid
                                                      • WHO subtypes
                                                        • Conventional OS (~ 75%)
                                                        • Telangiectatic OS (5-7%)
                                                        • Small cell OS (1-2%)
                                                        • Low-grade central OS (1-2%)
                                                        • Secondary OS
                                                        • Surface OS
                                                          • Parosteal OS (5%)
                                                          • Periosteal OS (1-2%)
                                                          • High-grade surface type (1%)
                                                      • Location
                                                        • Appendicular > > > axial skeleton (~ 10%)
                                                        • ~ 90% of conventional OS is metaphyseal
                                                        • ~ 75% of periosteal OS is diaphyseal
                                                        • Majority of OS occurs in femur (40-70%)
                                                          • Proximal tibia (16-25%) and proximal humerus (5-15%) are next most common for conventional OS
                                                        • Rarely soft tissue primary
                                                      • Morphology
                                                        • Aggressive osseous tumor with variable lytic and osteoblastic components and variable associated soft tissue mass
                                                          • Depending on tumor subtype and histologic variation
                                                    • Associated Syndromes

                                                      • Predisposition with
                                                        • Retinoblastoma gene mutation
                                                        • Li-Fraumeni syndrome
                                                        • Rothmund-Thompson syndrome
                                                        • Mutations of TP53 and MDM2 (murine double minute oncogene)
                                                        • Chromosomal heterozygosity at 3q, 13q, and 18q

                                                    IMAGING

                                                    • F-18 FDG PET/CT

                                                      • PET/MR

                                                        • F-18 NaF PET Bone Scan

                                                          • Nuclear Medicine Findings

                                                            • Imaging Recommendations

                                                              DIFFERENTIAL DIAGNOSIS

                                                                PATHOLOGY

                                                                • General Features

                                                                  • Staging, Grading, & Classification

                                                                    • Gross Pathologic & Surgical Features

                                                                      • Microscopic Features

                                                                        CLINICAL ISSUES

                                                                        • Presentation

                                                                          • Demographics

                                                                            • Natural History & Prognosis

                                                                              • Treatment

                                                                                DIAGNOSTIC CHECKLIST

                                                                                • Consider

                                                                                  Selected References

                                                                                  1. Hurley C et al: Comparison of (18) F-FDG-PET-CT and bone scintigraphy for evaluation of osseous metastases in newly diagnosed and recurrent osteosarcoma. Pediatr Blood Cancer. 63(8):1381-6, 2016
                                                                                  2. Kubo T et al: Prognostic significance of (18)F-FDG PET at diagnosis in patients with soft tissue sarcoma and bone sarcoma; systematic review and meta-analysis. Eur J Cancer. 58:104-11, 2016
                                                                                  3. Liu F et al: Performance of positron emission tomography and positron emission tomography/computed tomography using fluorine-18-fluorodeoxyglucose for the diagnosis, staging, and recurrence assessment of bone sarcoma: a systematic review and meta-analysis. Medicine (Baltimore). 94(36):e1462, 2015
                                                                                  4. Quartuccio N et al: Pediatric bone sarcoma: diagnostic performance of ¹⁸F-FDG PET/CT versus conventional imaging for initial staging and follow-up. AJR Am J Roentgenol. 204(1):153-60, 2015
                                                                                  5. Green JT et al: Osteogenic tumors of bone. Semin Diagn Pathol. 31(1):21-9, 2014
                                                                                  6. Luetke A et al: Osteosarcoma treatment - where do we stand? A state of the art review. Cancer Treat Rev. 40(4):523-32, 2014
                                                                                  7. Byun BH et al: Combination of 18F-FDG PET/CT and diffusion-weighted MR imaging as a predictor of histologic response to neoadjuvant chemotherapy: preliminary results in osteosarcoma. J Nucl Med. 54(7):1053-9, 2013
                                                                                  8. Fox MG et al: Osteosarcoma: review of the various types with emphasis on recent advancements in imaging. Semin Musculoskelet Radiol. 17(2):123-36, 2013
                                                                                  9. Hongtao L et al: 18F-FDG positron emission tomography for the assessment of histological response to neoadjuvant chemotherapy in osteosarcomas: a meta-analysis. Surg Oncol. 21(4):e165-70, 2012
                                                                                  10. Inaki A et al: Thallium-201 scintigraphy for the assessment of long-term prognosis in patients with osteosarcoma. Ann Nucl Med. 26(7):545-50, 2012
                                                                                  11. Cui J et al: Epigenetic changes in osteosarcoma. Bull Cancer. 98(7):E62-8, 2011
                                                                                  12. Dai X et al: Review of therapeutic strategies for osteosarcoma, chondrosarcoma, and Ewing's sarcoma. Med Sci Monit. 17(8):RA177-190, 2011
                                                                                  13. Kaste SC: Imaging pediatric bone sarcomas. Radiol Clin North Am. 49(4):749-65, vi-vii, 2011
                                                                                  14. Yarber JL et al: Targeted therapies in bone sarcomas: current approach and future directions. Expert Opin Investig Drugs. 20(7):973-9, 2011
                                                                                  15. Gorlick R et al: Osteosarcoma. J Bone Miner Res. 25(4):683-91, 2010
                                                                                  16. Ritter J et al: Osteosarcoma. Ann Oncol. 21 Suppl 7:vii320-5, 2010
                                                                                  17. Cheon GJ et al: Prediction model of chemotherapy response in osteosarcoma by 18F-FDG PET and MRI. J Nucl Med. 50(9):1435-40, 2009
                                                                                  18. Costelloe CM et al: 18F-FDG PET/CT as an indicator of progression-free and overall survival in osteosarcoma. J Nucl Med. 50(3):340-7, 2009
                                                                                  19. Gorlick R: Current concepts on the molecular biology of osteosarcoma. Cancer Treat Res. 152:467-78, 2009
                                                                                  20. Ottaviani G et al: The etiology of osteosarcoma. Cancer Treat Res. 152:15-32, 2009
                                                                                  21. Damron TA et al: Osteosarcoma, chondrosarcoma, and Ewing's sarcoma: National Cancer Data Base Report. Clin Orthop Relat Res. 459:40-7, 2007
                                                                                  22. McCarville MB et al: The cause and clinical significance of central tumor photopenia on thallium scintigraphy of pediatric osteosarcoma of the extremity. AJR Am J Roentgenol. 188(2):572-8, 2007
                                                                                  23. Nadel HR: Bone scan update. Semin Nucl Med. 37(5):332-9, 2007
                                                                                  24. Hayden JB et al: Osteosarcoma: basic science and clinical implications. Orthop Clin North Am. 37(1):1-7, 2006
                                                                                  25. Bacci G et al: Neoadjuvant chemotherapy for osteosarcoma of the extremities with metastases at presentation: recent experience at the Rizzoli Institute in 57 patients treated with cisplatin, doxorubicin, and a high dose of methotrexate and ifosfamide. Ann Oncol. 14(7):1126-34, 2003
                                                                                  26. Brenner W et al: PET imaging of osteosarcoma. J Nucl Med. 44(6):930-42, 2003
                                                                                  27. Ozcan Z et al: Correlation of 99mTc-sestamibi uptake with blood-pool and osseous phase 99mTc-MDP uptake in malignant bone and soft-tissue tumours. Nucl Med Commun. 22(6):679-83, 2001
                                                                                  28. Garcia R et al: Comparison of fluorine-18-FDG PET and technetium-99m-MIBI SPECT in evaluation of musculoskeletal sarcomas. J Nucl Med. 37(9):1476-9, 1996